Columbia resident overcomes challenges of Friedreich’s ataxia with newly-approved treatment

By Michelle-Renee Quinn

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    COLUMBIA, Missouri (KOMU) — One Columbia woman says a newly-approved treatment for her rare neuromuscular disorder has been life changing.

Skyclarys (omaveloxolone) was approved by the U.S. Food and Drug Administration in February for those diagnosed with Friedreich’s ataxia − a rare, inherited, degenerative disease that damages the nervous system, characterized by impaired coordination and walking. The treatment is now available for purchase.

Shelby Wright, a 27-year-old Columbia resident who has been living with Friedreich’s ataxia for more than half her life, recently started taking the new medication. She said it has given her perspective.

“When I think about the people who died without a treatment because of this disease, I feel really thankful to be part of the first-ever treatment,” Wright said.

Wright was diagnosed with Friedreich’s ataxia at the age of 12. Growing up, she said she ignored the diagnosis and went on with her life.

“As things started progressing, staying up later got harder, walking got harder, I basically adapted as much as I needed to,” she said.

Friedreich’s ataxia often strikes between the ages of 10 to 15. Some symptoms include poor muscle control that causes clumsy voluntary movements, ataxia and proprioception; the sense that lets us perceive the location, movement and action of parts of the body, according to the FDA.

When it comes to daily activities and fun, Wright said one thing the disorder took away was the ability to ride her dirt bike.

“That was one of the hardest things,” she said.

Due to heart problems from her childhood, Wright struggled to ride a bike, so her family purchased a dirt bike that she loved to ride.

“When I was 12 and diagnosed with FA [Friedreich’s ataxia], the doctor looked at me and said, ‘You’re not going to be able to ride a dirt bike any longer,'” Wright said.

Friedreich’s ataxia causes gradual damage to the spinal cord, peripheral nerves and the brain, which can result in uncoordinated muscle movement, poor balance, difficulty walking, changes in speech and swallowing, and a shortened lifespan.

So far, Wright said she hasn’t experienced any side effects that her pharmacist warned her of, like nausea or fatigue.

Wright says this treatment should elongate her life by slowing the progression of the disorder.

“For it to be the first-ever treatment is really something, and something really historical that I am so thankful to be part of,” Wright said.

Wright’s message to those living with Friedreich’s ataxia is to explore all the possibilities in life, adapt and overcome as much as possible.

“I know that can be difficult because I run into difficulties all the time, it’s not the easiest thing,” she said. “There’s just some things that you cannot do, but there is always things that you can do.”

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